| Coprogen_oxidas | |||||||||
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| coproporphyrinogen iii oxidase from leishmania major | |||||||||
| Identifiers | |||||||||
| Symbol | Coprogen_oxidas | ||||||||
| Pfam | PF01218 | ||||||||
| InterPro | IPR001260 | ||||||||
| PROSITE | PDOC00783 | ||||||||
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Coproporphyrinogen-III oxidase, mitochondrial is an enzyme that in humans is encoded by the CPOX gene.[1][2][3] A genetic defect in the enzyme results in a reduced production of heme in animals. The medical condition associated with this enzyme defect is called hereditary coproporphyria.[4] [5]
It is an enzyme involved in the sixth step of porphyrin metabolism it catalyses the oxidative decarboxylation of coproporphyrinogen III to proto-porphyrinogen IX in the haem and chlorophyll biosynthetic pathways.[2][6] The protein is a homodimer containing two internally bound iron atoms per molecule of native protein.[7] The enzyme is active in the presence of molecular oxygen that acts as an electron acceptor. The enzyme is widely distributed having been found in a variety of eukaryotic and prokaryotic sources.
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This article incorporates text from the public domain Pfam and InterPro IPR001260